Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The French-American and British (FAB) classification of 62 consecutive cases of acute myeloid leukemia was undertaken. AML-M2 was the commonest FAB type (32.26%), followed by M1 and M4 (22.58% each), M5 (8.6%) and M6 and M7 (1.61% each), respectively. One of the patients was diagnosed as AML-MO (not a FAB type). The mean age of M1, M2, M3 and M5 cases was between 25 and 29 years, whereas in M4 patients it was 45.6 years. AML-M2, M4 and M5 were commoner in males, M1 in females and M3 equal in both sexes. Feeling of weakness, easy fatiguability and pallor were invariably present in all FAB types. All the patients of M1 and M5, 85% of M2, 64% of M4 and 50% of M3 presented with fever. Bleeding manifestations were most frequent in M3 cases followed by M5, M1, M4 and M2, respectively. Hepatomegaly and splenomegaly were relatively less prominent features in M3 as compared to other FAB types. Amongst the haematological parameters, anaemia was more severe in M1, leucocytosis in M2 and thrombocytopenia in M3 cases as compared to other FAB types.
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PMID:Acute myeloid leukemia-FAB classification and its correlation with clinico-haematological features. 811 48

With an increasing number of elderly patients presenting with acute myeloid leukaemia and with recent reports of worthwhile remission rates and survival times following treatment with intensive chemotherapy, there is a pressing need to identify criteria to assist in the selection of appropriate therapy for elderly patients. We have performed multivariate Cox proportional hazard regression analysis of data prospectively collected during the treatment of 104 patients aged 60 and over treated in a multi-centre study with a standardized regimen of mitozantrone and cytosine arabinoside for induction and consolidation. Four readily available parameters, namely urea, performance status, peripheral blood blast count and presence of hepatomegaly, were identified from which a prognostic model to predict survival has been developed. The model was found to be accurate in predicting survival in the cohort of patients from which it was developed, but needs to be validated in a further test population studied prospectively. Its simplicity suggests that it may be particularly useful in the selection of elderly patients with AML most likely to benefit from intensive chemotherapy.
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PMID:Prognostic factors in elderly patients with acute myeloid leukaemia: development of a model to predict survival. 828 Jun 3

A 35-yr-old female presented with symptoms of obstructive jaundice. Liver biopsy, bone marrow aspiration, and biopsy revealed systemic mastocytosis and acute myeloid leukemia. The liver biopsy specimen showed infiltration of mast cells within portal tracts with periductal and portal edema, irregularity of interlobular duct epithelium, and centrizonal cholestasis. Endoscopic retrograde cholangiography was normal. Following chemotherapy treatment with idarubicin and cytarabine for seven days for AML, the bilirubin levels continued to increase for two weeks and then decreased, reaching normal levels in two months. Infiltration of mast cells in the liver leads to hepatomegaly, liver function abnormality and rarely portal hypertension. Intrahepatic cholestasis due to systemic mastocytosis has never been reported. We report a rare case of systemic mastocytosis causing intrahepatic cholestasis that resolved with remission of AML following chemotherapy.
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PMID:Intrahepatic cholestasis due to systemic mastocytosis: a case report and review of literature. 921 99

To know the clinical characteristics and the prognostic factors of hyperleukocytic acute leukemia, we reviewed 244 patients with acute leukemia associated with hyperleukocytosis. Restrospective analysis and control study were used. Hyperleukocytosis occured in 8.5% of patients with acute leukemia. Hyperleukocytosis in ALL was more common than that in AML. Among AML with hyperleukocytosis, M5 subtype was the most. Hepatomegaly, splenomegaly, lymphadenopathy, DIC and CNSL were more frequent in hyperleukocytosis group. The complete remission rate was 41.4% for patients with hyperleukocytosis versus 54.2% for patients with non-hyperleukocytosis. The early mortality rate was significantly increased in hyperleukocytic patients (23.8%) as compared to the nonhyperleukocytic group (11.1%). Intracranial hemorrhage was the main cause of early death. The high risk factors of early death were: hemoglobin < or = 40 g/L, blood platelet < or = 30 x 10(9)/L, DIC, infection, CNSL at presentation. Acute leukemia with hyperleukocytosis has poor prognosis. Especially, acute myeloid leukemia with hyperleukocytosis must be taken seriously because of high early mortality rate.
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PMID:[244 patients with hyperleukocytic acute leukemia. Shanghai Leukemia Cooperation Group]. 1043 59

There has not been a reported series of children with therapy-induced myelodysplastic syndrome/acute myeloid leukemia (tMDS/tAML) who were treated systematically. This paper describes 24 children with tMDS/tAML who were assigned randomly to standard- or intensive-timing induction on protocol CCG 2891. Presenting features and outcomes of those children were compared with those of 960 patients with de novo MDS (62 patients) or AML (898 patients). Children with tMDS/tAML were older at presentation (P =.015), had lower white blood cell counts (P =.01), and were more likely to have MDS (21% vs 7%) (P =.02) and trisomy 8 (P =.06). Fewer had hepatomegaly (P =.02), splenomegaly (P =.03), hepatosplenomegaly (P =.02), or classic AML translocations [t(8;21), t(15;17), 16q22; P =.02]. They had a poorer induction rate (50% vs 72%, P =.016), overall survival (26% vs 47% at 3 years, P =.007), and event-free survival (21% vs 39% at 3 years, P =.023). Disease-free survival after achieving remission was similar (45% vs 53%, P =.868). Children with tMDS/tAML who received intensive-timing induction had better outcomes than those who received standard-timing induction (overall survival 32% vs 0%, P =.54). In this study, the latency period to development of tMDS/tAML was the same for presumed alkylator-induced as for topoisomerase-induced myeloid leukemia. The findings of this study confirm that most children with tMDS/tAML have disease resistant to current therapies. Standard-timing induction appears less effective for this population.
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PMID:Acute myeloid leukemia and myelodysplastic syndrome in children treated for cancer: comparison with primary presentation. 1209 32

A 40 year old man presented with abdominal pain, jaundice, weight loss, and hepatosplenomegaly. Liver function tests revealed cholestatic jaundice and a computed tomography scan showed an enlarged liver, with a normal biliary tree. Liver biopsy showed diffuse infiltration by neutrophils, monocytoid cells, and blasts. Peripheral blood film and bone marrow were consistent with acute myeloid leukaemia. After treatment with chemotherapy using an acute myeloid leukaemia protocol (UK Medical Research Council AML-12), there was complete resolution of jaundice and the patient went into complete molecular remission.
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PMID:Acute myeloid leukaemia presenting as cholestatic hepatitis. 1511 66

To evaluate the liver function, splenomegaly and related factors in the newly diagnosed acute leukemia patients. One hundred of fifty eight acute leukemia patients admitted in our hospital from March 2003 to April 2006 were studied. The related factors such as peripheral WBC count, bone marrow blasts, peripheral blasts, sex, age, AML, ALL affecting the liver function and splenomegaly were evaluated. Sixty two (39.24%) patients presented with splenomegaly. Twelve (7.59%) patients presented with hepatomegaly. Serum ALT was elevated in 54 (34.17%) patients. Similarly, serum AST, GGT, ALP, and Direct bilirubin were elevated in 26 (16.45%), 32 (20.25%), 20 (12.65%), and 22 (13.92%) patients, respectively. Low serum albumin was found in 40 (25.31%) patients. PT was prolonged in 62 (39.24%) patients. Statistical study shows that there is a relation between high WBC counts and elevated serum ALT (P<0.05) and high WBC counts and splenomegaly (P<0.05). Acute leukemia patients with leukocytosis are more prone to develop abnormal liver function and splenomegaly.
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PMID:Abnormal hepatic function and splenomegaly on the newly diagnosed acute leukemia patients. 1834 Mar 67

This study evaluates the clinical and laboratory data of children with secondary hemophagocytic lymphohistiocytosis (sHLH) related to malignancy. Charts of patients who met the diagnostic criteria for sHLH associated with malignancy between January 2000-2006 at six different hospitals in Turkey were reviewed retrospectively. The diagnosis of HLH had been established by bone marrow aspiration in 27 patients, cerebrospinal fluid and bone marrow aspiration in one patient and lung-liver biopsy in another. Twenty-nine children were diagnosed as having sHLH related to malignancy. Twenty cases (18 ALL and 2 AML) with acute leukemia (10 girls/10 boys, median age: 8 years [3-14 years]) were found to have sHLH. Five patients with acute leukemia had HLH at the time of diagnosis (Group 1a), and 15 patients with acute leukemia were diagnosed as having sHLH during therapy (Group 1b), namely reactive sHLH associated with the chemotherapy. Nine patients, including two cases each of rhabdomyosarcoma, neuroblastoma, Hodgkin disease, and non-Hodgkin lymphoma (NHL) and one case with Langerhans cell histiocytosis, were diagnosed as having concomitant hemophagocytosis at the initial evaluation of the tumor (Group 2). Fever, anemia, and hypertriglyceridemia were present in all sHLH cases of all three groups. Hepatomegaly was detected in 60.0%, 73.3%, and 88.8% of the three groups, respectively. Splenomegaly was more frequent in patients of Groups 1a (60.0%) and 2 (88.8%) than in those of Group 1b, the reactive ones (13.3%). Hypofibrinogenemia was detected in all patients of Group 1a and Group 2. Low level of fibrinogen was present in 91.6% of patients in Group 1b. All patients in Group 1b (100%) had neutropenia and thrombocytopenia. Neutropenia was found at rates of 60.0% and 55.5% in Group 1a and Group 2, respectively. Thrombocytopenia was detected in 80.0% of patients in Group 1a and 77.7% in Group 2. The overall mortality rate was 34.4% (10 cases) in our series of 29 children with sHLH; 50% of deaths were directly attributable to HLH. Pediatric malignancy-associated HLH patients have been commonly described as case presentations or in a review of the literature. We believe that our cohort, compiling 29 children regarding the association between malignancy and HLH, will be useful for pediatricians who are interested in this still mysterious topic.
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PMID:Malignancy-associated hemophagocytic lymphohistiocytosis in pediatric cases: a multicenter study from Turkey. 1981 62

Leukemias are the commonest childhood malignancy in West Bengal. This study was undertaken on 75 children at NRS Medical College, West Bengal to determine the distribution of signs and symptoms of leukemia and to identify unusual clinical features. After obtaining clinical history, physical examination, hematological and radiological investigations were performed. Acute lymphoblastic leukaemia (ALL, 72%) was the commonest followed by acute myeloid leukaemia (AML, 18.7%). Common symptoms and signs were fever (85.3%), pallor (64%), hepatomegaly (72%), splenomegaly (60%) and lymphadenopathy (50.7%). The uncommon signs and symptoms were abdominal pain (9.3%), joint pain (9.3%), hematemesis and malena (8%), diarrhea (5.3%), proptosis (2 cases), dysphagia, mediastinal mass and parotid swelling (1 case each). Uncommon clinical presentations lead to delay in diagnosis in some cases. Awareness of uncommon signs and symptoms of childhood leukemia together with laboratory tests may help in earlier diagnosis and proper management of the patients.
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PMID:Childhood acute leukemia in West Bengal, India with an emphasis on uncommon clinical features. 2059 69

AML-M6 has a peak incidence in the seventh decade with slight male preponderance, and can also present at a younger age. The usual features are anaemia, thrombocytopenia, malaise, fatigue, easy bruising, epistaxis and petechiae. Splenomegaly may occur in 20-40 % of the cases but massive splenomegaly is rare presentation and have been only reported once in humans and once in animals. A 22 year Asian female, presented with fatigue, pallor, mild jaundice, exertional dyspnoea, epigastric pain, tender right hypochondrium and massive splenomegaly. Investigations revealed anaemia and thrombocytopenia, tear drop cells, basophilic stippling, piokilocytosis and anisochromia; increased uric acid and LDH. Abdominal ultrasound showed enlarged liver (22cm) and spleen (20cm). Bone marrow aspiration revealed 51% erythroid and 24% non-erythroid precursors, depressed leukopoeisis and megakarypoeisis. Erythroblasts were PAS and CD71 positive and also reacted to Antihaemoglobin-Antibody. This report highlights characteristic features and diagnostic criteria of erythroleukaemia, differential diagnosis of massive splenomegaly and their rare association.
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PMID:Massive splenomegaly in acute erythroid leukaemia (FAB Class-M6): an unusual presentation. 2313 97


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